Underestimated abdominal vascular pathology in a patient with Takayasu arteritis.

Objective: To describe a 31-year-old female with symptomatic Takayasu disease who was operated for aortic valve replacement. Although she had no preoperative abdominal vascular symptoms, she died on the first postoperative day due to extensive ischemia bowel syndrome.

Methods: Echocardiography and computed tomography revealed progressive dilatation and thickening of the ascending aorta, severe aortic regurgitation and diminished left ventricular function from 1998 onwards. In 2000 she was operated and a prosthesis was placed end-to-end distally of the sinutubular junction with combined reduction plasty of this junction. After the operation, in time echocardiography and computed tomography showed progressive irregularities and dilatation of the thoracoabdominal aorta and progressive aortic regurgitation. A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned. At the time of the reoperation in September 2004 the Takayasu inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase. The aortic valve was uneventfully replaced by a mechanical valve.

Results: Medical treatment for Takayasu disease never resulted in the patient in completely normal blood values of white blood cell count, C-reactive protein or erythrocyte sedimentation rate. In August 2004, she was admitted because of severe non-specific thoracic pain and hypertension. During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension. Although the computed tomography revealed severe stenosis of the superior mesenteric artery and the celiac trunk, she was free of abdominal complaints and without further abnormal laboratory findings. One day after the aortic valve replacement a dramatic increase of transaminase and lactate-dehydrogenase with extreme metabolic acidosis appeared. Urgent abdominal surgery was performed and extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia, no surgical interventions were optional. The patient died one day after abdominal exploration.

Conclusions: We conclude that in Takayasu disease scheduled for on pump cardiac surgery, vascular workup should be done, and interventional treatment of asymptomatic but potentially critical lesions should be considered.